lateral aspects associated with preservation of the most dorsal portion of the arch; Type D, the complete absence of the posterior arch with a persistent posterior tubercle; and Type E, the complete absence of the posterior arch and posterior tubercle. 2 Few cadaveric, imaging, and case studies have been reported on the variations of such C-1 arch defects, and their clinical significance for neurosurgical practice is unclear. We therefore investigated the incidence of these congenital defects in the cervical spine on CT studies, in fresh human cadaveric cervical spines
Mehmet Senoglu, Sam Safavi-Abbasi, Nicholas Theodore, Nicholas C. Bambakidis, Neil R. Crawford and Volker K. H. Sonntag
A surgical approach to aplasia cutis congenita
Peter J. Lynch and Edgar A. Kahn
. E. Congenital scalp defects in twin sisters. Am. J. Dis. Child. , 1965, 110: 293–294. 8. Ingalls , N. W. Congenital defects of the scalp. Studies in the pathology of development III. Am. J. Obstet. Gynec. , 1933 , 25 : 861 – 873 . Ingalls , N. W. Congenital defects of the scalp. Studies in the pathology of development III. Am. J. Obstet. Gynec. , 1933, 25: 861–873. 9. Johnsonbaugh , R. E. , Light , I. J. , and Sutherland , J. M. Congenital scalp defects in father and son. Am. J. Dis. Child
Harry M. Rogers, Don M. Long, Shelley N. Chou and Lyle A. French
symptoms and signs, and findings at surgery, with the possible exception of a high association of bladder involvement with lesions of the filum terminale in their series. Bladder dysfunction (enuresis) was the prominent symptom in the one patient in whom the tumor was attached primarily to the filum. Discussion The exact pathogenesis of spinal cord lipomas is unclear although the high incidence of associated congenital defects, the age of onset, and location of the tumors suggest a developmental origin. This is especially evident in those with a midline
Krishna Kumar Bisaria
✓ Congenital defects of the tentorium cerebelli were observed in 16 of 90 cadaver cranial cavities examined. These consisted of tentorial dural bands in two, a hole in one case, transverse ridges in eight cases, and an aperture in five cases. In one specimen, the trochlear nerve made a spiral turn around the tentorial band before pursuing its forward course.
Dennis D. Nichols and Anthony G. Bottini
defect. By rotating skull and pericranial flaps under an intraoperatively expanded scalp an excellent cosmetic result is achieved without a staged procedure. Protection of the underlying sinus and brain is also completed in a timely manner, obviating concern about prolonged hospitalization costs and the risks of conservative treatment. 7, 11, 36 References 1. Anderson NP , Novy FG : Congenital defect of scalp. Arch Dermatol Syph 46 : 257 – 263 , 1942 Anderson NP, Novy FG: Congenital defect of scalp. Arch Dermatol Syph 46
Timothy W. Vogel, Sunil Manjila and Alan R. Cohen
Giant occipitoparietal encephaloceles are rare forms of neurodevelopmental defects whose etiologies remain uncertain. Their occurrence can lead to variable neurological outcomes depending on the extent of cerebral cortex involved and the ability to repair the defect. In addition, encephaloceles may be associated with various genetic syndromes and familial inheritance. Here, the authors describe a unique constellation of malformations associated with the case of a giant occipitoparietal meningoencephalocele with herniation of cortical tissue and continuity with the ventricular system. The patient had a cleft lip and palate, hemivertebrae of the thoracic spine, a patent ductus arteriosus, a ventricular septal defect, and coarctation of the aorta. To identify the genetic underpinnings of these malformations, fluorescence in situ hybridization and microarray analysis were performed and revealed an 80.65-kb gain within chromosome band 2p11.2. Duplications of this region involving RMND5A, whose product contains a C-terminal to lis homology (LisH) domain, have not previously been associated with a defined phenotype but may present insight into encephalocele formation. Surgical repair and follow-up for the neurological malformations are also discussed.
Mitchel Seruya, Albert K. Oh, Gary F. Rogers, Michael J. Boyajian, John S. Myseros, Amanda L. Yaun and Robert F. Keating
Controlled hypotension is routinely used during open repair of craniosynostosis to decrease blood loss, although this benefit is unproven. In this study the authors analyzed the longitudinal relationships between intraoperative mean arterial pressure (MAP) and calculated blood loss (CBL) during frontoorbital advancement (FOA) for craniosynostosis.
The authors reviewed the records of infants with craniosynostosis who had undergone primary FOA between 1997 and 2009. Anesthesia records provided preoperative and serial intraoperative MAP. Interval measures of CBL had been determined during the course of the operation. The longitudinal relationships between MAPmean, MAPchange, and CBLchange were assessed over the same time interval and compared between adjacent time intervals to determine the directionality of associations.
Ninety infants (44 males and 46 females) underwent FOA at a mean age and weight of 10.7 ± 12.9 months and 9.0 ± 7.0 kg, respectively. The average intraoperative MAP was 56.1 ± 4.8 mm Hg, 22.6 ± 12.1% lower than preoperative baseline. A negative correlation was found between CBLchange and MAPmean over the same interval (r = −0.31, p < 0.05), and an inverse relationship was noted between CBLchange of the previous interval and MAPchange of the next interval (r = −0.07, p < 0.05). Finally, there was no significant association between MAPchange of the previous interval and CBLchange of the next interval.
Calculated blood loss demonstrated a negative correlation with MAP during FOA. Directionality testing indicated that MAP did not affect intraoperative blood loss; instead, blood loss drove changes in MAP. Overall, these findings challenge the benefit of controlled hypotension during open craniofacial repair.
Richard L. Rapport II, Robert C. Dunn Jr. and Fareeda Alhady
✓ Eighteen cases of anterior encephalocele treated at the University of Malaya Hospital between 1970 and 1980 are discussed, and the literature concerning this defect is reviewed. A detailed analysis of the microscopic abnormalities present in the surgical specimens is included, along with the relevant radiographic and demographic data. Anterior encephalocele is more common in Southeast Asia than elsewhere. The possible ethnographic and geographic implications are presented, as well as a discussion of the relevant embryology, in attempting to define possible etiologies for this malformation. The authors' surgical approach to the repair of this defect and reasons for preferring a transcranial, intradural approach are described. Potential complications are enumerated.
Kimberly M. Hamilton, Andrea L. Wiens and Daniel H. Fulkerson
E ncephaloceles are acquired or congenital defects in which intracranial contents herniate through a defect in the calvaria. The risk of encephalocele formation is associated with both genetic and environmental factors. The anatomical location varies with geographical region, with occipital encephaloceles common in Western Europe and North America, and anterior encephaloceles common in Southeast Asia, Russia, and central Africa. The vast majority of encephaloceles occur at or near the midline. A number of theories have been proposed to explain the
Case report and review of the literature
Gregory C. Dowd, Timothy B. Molony and Rand M. Voorhies
delineated the multiple causes of nonspontaneous pneumocephalus in 1926. 2 Spontaneous pneu-mocephalus occurring in the absence of pathological conditions or iatrogenic interventions was first described in 1954 by Jelsma and Moore. 9 Markham 13 made the study of all forms of pneumocephalus his life's work and published an extensive series and literature review in 1967. Interestingly, spontaneous pneumocephalus represented just 0.6% of his 295 cases. Spontaneous otogenic pneumocephalus can occur when a congenital defect in the pneumatized bone surrounding the middle ear